Pregnancy in Women With Congenital Heart Disease and Pulmonary Hypertension

Quick Takes

  • Women with mild pulmonary hypertension (PH) had lower risks of adverse maternal cardiac and obstetric events as well as offspring events as compared with those in the moderate-to-severe PH subgroup.
  • For women with congenital heart disease (CHD)-related PH, follow-up with a multidisciplinary team and strict antenatal supervision were both associated with lower rates of maternal cardiac complications.
  • BNP level and NYHA functional class may help to risk stratify women with CHD and PH.

Study Questions:

What are outcomes for pregnancy in women with congenital heart disease (CHD) with and without pulmonary hypertension (PH)?

Methods:

Outcomes for women with CHD were evaluated retrospectively from 1993 to 2016 and prospectively from 2017 to 2019 at seven tertiary centers in China. Diagnosis of PH was based on echocardiogram or catheterization. The incidence of maternal death, cardiac complications, and obstetric and offspring complications was compared for women with CHD and no PH, mild PH, and moderate-to-severe PH.

Results:

A total of 2,220 pregnant women with CHD had completed pregnancies. PH associated with CHD was identified in 729 women. Mild PH (right ventricle [RV] to right atrium [RA] gradient 30-50 mm Hg) was present in 398 women, while moderate PH (RV to RA gradient >50 mm Hg) was present in 331. Maternal mortality occurred in one (0.1%) woman with no PH, no women with mild PH, and 19 (5.7%) women with moderate-to-severe PH. Overall, patients with mild PH had better maternal outcomes than those with moderate-to-severe PH. Obstetric complications occurred in 5.3% of women with mild PH as compared with 15.7% of women with moderate-to-severe PH (p < 0.001). B-type natriuretic peptide (BNP) >100 ng/L and New York Heart Association (NYHA) class III-IV were independently associated with adverse maternal cardiac events, while follow-up with a multidisciplinary team and strict antenatal supervision were protective.

Conclusions:

Women with CHD and mild PH appear to have better pregnancy outcomes as compared with those with moderate-to-severe PH.

Perspective:

Pregnancy in women with PH has historically been associated with extremely high (approaching 50%) mortality, although outcomes have improved in recent years. This multicenter study showed improving maternal outcomes and demonstrated the heterogeneity of this disease process, with significantly better outcomes for women with CHD and mild PH as compared with those with moderate-to-severe PH. Pregnancy termination or miscarriages occurred in a relatively high proportion of pregnancies with moderate-to-severe PH (494 out of 825), which raises the possibility that sicker patients underwent termination. Additionally, the overall favorable outcomes seen in this study occurred in the setting of an extremely high rate of Caesarean delivery (83-92% based on severity of PH). The optimal mode of delivery for patients with CHD and PH remains unclear. Overall, this study suggests a role for individualized counseling for patients regarding risks, and meticulous, multidisciplinary team-based care for patients with CHD and PH contemplating or undergoing pregnancy.

Clinical Topics: Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Pulmonary Hypertension, Echocardiography/Ultrasound, Hypertension

Keywords: Abortion, Spontaneous, Catheterization, Counseling, Echocardiography, Heart Defects, Congenital, Heart Failure, Hypertension, Pulmonary, Natriuretic Peptide, Brain, Patient Care Team, Pregnancy, Pregnancy Outcome, Risk Assessment, Secondary Prevention, Women


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